Epidermal nevus syndrome
Epidermal nevus syndrome (ENS), also known as Solomon's syndrome or Feuerstein and Mims syndrome, represent a group of distinct disorders related to the presence of epidermal nevi and extracutaneous anomalies. It is a syndrome linked to mosaicism, with many of them occurring exclusively sporadically, but some with known hereditary traits 1.
There are nine well defined epidermal nevus syndromes:
- Schimmelpenning syndrome
- phacomatosis pigmentokeratotica
- nevus comedonicus syndrome
- Becker nevus syndrome
- angora hair nevus syndrome
- Proteus syndrome
- type 2 segmental Cowden disease
- CHILD syndrome
- FGFR3 epidermal nevus syndrome
CLOVE syndrome may also present with epidermal nevi.
History and etymology
Solomon et al. described ENS as one single entity characterized by the coexistence of epidermal nevi and extracutaneous anomalies 1.
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